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Table of Contents
CASE REPORT
Year : 2019  |  Volume : 7  |  Issue : 4  |  Page : 131-133

A rare case of truncus arteriosus in the elderly


Department of General Medicine, M.R. Medical College, Kalaburgi, Karnataka, India

Date of Submission11-Jun-2019
Date of Decision16-Jul-2019
Date of Acceptance19-Jul-2019
Date of Web Publication18-Oct-2019

Correspondence Address:
Dr. Sangram Biradar
Department of General Medicine, M.R. Medical College, Kalaburgi, Karnataka
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/AJIM.AJIM_6_19

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  Abstract 

Truncus arteriosus (TA) is a rare cyanotic congenital heart disease, with average survival up to 5 weeks to 6 months. There are factors such as pulmonary arterial hypertension and hypoplasia of the pulmonary branches that are deleterious for life in some cardiovascular diseases. The natural history of the unrepaired defect is not very well known, and is backed by only a handful of such reported cases. We report this very rare case of an uncorrected TA surviving into adulthood and bearing four uneventful childbirths.

Keywords: Congenital heart diseases, truncal regurgitation, truncus arteriosus, truncus arteriosus in adults, unrepaired truncus arteriosus


How to cite this article:
Choudhary VH, Biradar S, Hirapur IS, Chanda SS. A rare case of truncus arteriosus in the elderly. APIK J Int Med 2019;7:131-3

How to cite this URL:
Choudhary VH, Biradar S, Hirapur IS, Chanda SS. A rare case of truncus arteriosus in the elderly. APIK J Int Med [serial online] 2019 [cited 2019 Dec 5];7:131-3. Available from: http://www.ajim.in/text.asp?2019/7/4/131/269570




  Introduction Top


Truncus arteriosus (TA) is a rare congenital cardiac malformation in which a single common artery arises from the heart by means of a single semilunar truncal valve and supplies the systemic, pulmonary, and coronary circulations,[1]

Because of the increased blood flow in the pulmonary circulation leading to heart failure, there is a high rate of early mortality, thus explaining the particularly poorer outcomes in Types I, II, and III, wherein 80% of the babies do not survive beyond infancy, while Type IV with low pulmonary blood flow leads to a comparatively longer survival, with the longest surviving case reported at the seventh decade.[2]

We report the case of a 55-year-old female with uncorrected TA, a rarity of the associated survival into adulthood and bearing four children.


  Case Report Top


A 55-year-old female patient presented with poor exercise tolerance, cyanosis, and transient loss of consciousness, with a childhood history of repeated lower respiratory infections.

The patient had progressive breathlessness from Class II to IV over the last 1 year; she had cyanosis; Grade 3 clubbing, with a pulse rate of 110; blood pressure of 110/40 high-volume pulse; regular, collapsing pulse with wide pulse pressure; dancing carotids; positive Landolfi sign; pulsatile nail bed (Quincke's sign), and pistol shot femoral pulse (Palfrey sign).

The apex beat was in the 7th intercostal space (ICS) 1 cm lateral to the midclavicular line, shifted down and out (suggesting left ventricular hypertrophy) with palpable diastolic thrill in the left 2nd and 3rd ICS, Grade 3 parasternal heave (suggesting right ventricular hypertrophy [RVH]); there was a high-pitch decrescendo early diastolic murmur Grade 4 present along the lower left sternal border radiating to the axilla (suggestive of regurgitation through the truncus) and a palpable s2 in the pulmonary area with loud p2 (suggestive of pulmonary hypertension).

Electrocardiogram has standardization 25 mm/s 10 mm/mV, rhythm sinus, rate 95, PR interval 198, Qrs 100 ms, Qt/Qtc 330/415, P waves 140 ms, Qrs voltage of S wave more than R wave in lead v4, No abnormal Q waves, normal St segment, normal T waves, No U waves f/s/o sinus rhythm, with biatrial enlargement, RVH, LVH (Cornell voltage criteria), poor r wave progression [Figure 1].
Figure 1: Electrocardiogram

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Two-dimensional echo showed congenital heart disease with situs solitus, large perimembranous ventricular septal defect (VSD) [Figure 2] with a bidirectional shunt, and a large TA [Figure 3] and [Figure 4] with severe regurgitation (eccentric jet) with normal biventricular function, with pulmonary arterial systolic pressure of 69 mmHg.
Figure 2: Parasternal long-axis view on echo showing truncus arteriosus along with ventricular septal defect

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Figure 3: Five-chamber view on echo with prominent truncus

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Figure 4: Five-chamber view on echo with prominent truncus

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Cardiac magnetic resonance imaging (MRI) showed Type 1 TA [Figure 5], [Figure 6], [Figure 7] in accordance with the Collet and Edward classification (Type I: aorta and main pulmonary arteries share a common arterial trunk, Type II: right and left pulmonary arteries arise separately from the posterior part of the trunk, Type III: separate origins of the pulmonary arteries from the lateral aspect of the trunk, and Type IV: neither pulmonary arterial branch arising from the common trunk, with the lungs supplied by collaterals [pseudotruncus]).
Figure 5: Axial section of magnetic resonance imaging showing the main pulmonary trunk with the ascending aorta and descending aorta

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Figure 6: Axial section of magnetic resonance imaging showing truncus arteriosus

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Figure 7: Axial section of magnetic resonance imaging showing the main pulmonary trunk with both atria

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  Discussion Top


TA, synonymous with common arterial trunk and common aorticopulmonary trunk, accounts for 0.7%–1.4% of all congenital heart diseases in live-born infants (incidence of 0.03–0.056/1000 live births).[3]

Most of such patients die within 5 weeks to 6 months. Survival into adulthood is rare, with the longest surviving case reported at the seventh decade.[4]

There is no striking sex difference in its incidence although most series have a male predominance.

Almost all such reported patients had pulmonary hypertension, and it was concluded that until now, we do not know the actual survival time of adult patients with complex congenital heart disease, such as TA.[5] Collett and Edward (1948) classified TA into four types. In Type 1, a single pulmonary trunk arises from the TA just distal to the truncal valve. In Types 2 and 3, the pulmonary trunk is absent, and the two pulmonary branches arise from the dorsal wall of the truncus (Type 2) or from the side of the truncus (Type 3). In Type 4, also called pseudotruncus, pulmonary arteries are absent, and the pulmonary circulation is supplied by the major aortopulmonary collateral arteries arising from the descending aorta. Type 4 now corresponds to pulmonary atresia with VSD.

The reason for the survival of a patient with unrepaired TA is still not properly understood; some hypotheses report that the presence of increased pulmonary vascular resistance during the 1st week of life decreases the development of heart failure, which is the main cause of early mortality.[6]

Due to the rarity of the survival of patients with unrepaired TA, the reason for the survival of the few cannot be ascertained, and the factors responsible for delay in the development of pulmonary hypertension are unknown.


  Conclusion Top


Our case is unique in terms of it being one of the few cases of unrepaired Collet Type I TA surviving to adulthood and bearing four pregnancies.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Mittal SK, Mangal Y, Kumar S, Yadav RR. Truncus arteriosus type 1: A case report. Indian J Radiol Imaging 2006. p. 229-31.  Back to cited text no. 1
    
2.
Venkatraman D, Bhandari M, Mukhopadhyay T, Majumder B, Bera D. A rare form of presentation of truncus arteriosus in adult. Nig J Cardiol 2015;12:148-50.  Back to cited text no. 2
  [Full text]  
3.
Cifarelli A, Ballerini L. Truncus arteriosus. Pediatr Cardiol 2003;24:569-73.  Back to cited text no. 3
    
4.
Perloff JK, Marelli AJ, editors. Perloff's Clinical Recognition of Congenital Heart Disease. 6th ed. Philadelphia: Elsevier. 2012.  Back to cited text no. 4
    
5.
Espínola-Zavaleta N, Muñoz-Castellanos L, González-Flores R, Kuri-Nivón M. Spanish. Arch Cardiol Mex 2008;78:210-6.  Back to cited text no. 5
    
6.
Abid D, Daoud E, Ben Kahla S, Mallek S, Abid L, Fourati H, et al. Unrepaired persistent truncus arteriosus in a 38-year-old woman with an uneventful pregnancy. Cardiovasc J Afr 2015;26:e6-8.  Back to cited text no. 6
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]



 

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